What is cystic fibrosis?

Cystic fibrosis is a rare condition that affects how the lungs work and makes it difficult for the body to digest food. A baby inherits cystic fibrosis through his genes, which means he is born with it.

Cystic fibrosis is caused by a faulty gene that controls the way salt is absorbed by the body. Too much salt and not enough water is allowed to pass into the body's cells. This turns the secretions, which normally lubricate our organs, into a thick, sticky mucus. This mucus blocks the airways in the lungs and clogs the digestive system.

It's a condition that causes lots of breathing problems. Colds and chest infections can pose a particular danger to children with the condition, and digesting food and drink is difficult for them. Children with cystic fibrosis can be quite small for their age and, even if they eat well, their growth can be slow (Cystic Fibrosis Trust 2011: 8).

However, although cystic fibrosis is a life-long condition, treatments and understanding of it are improving all the time.

How will I know if my baby has cystic fibrosis?

For the check, your midwife will take a few drops of blood from your baby's heel. The blood is tested in a laboratory for cystic fibrosis and other inherited conditions, such as sickle cell anaemia.

If cystic fibrosis is suspected, your doctor will carry out a sweat test. This will look for levels of salt in your baby's sweat, which will be high if he has cystic fibrosis (Cystic Fibrosis Trust: 32, NHS 2011).

If your baby has not been tested and he has the condition, it may soon become clear that he is unwell. In about one in 10 newborns with cystic fibrosis, the greenish poo, or meconium, which passes through a baby's bowels just after birth, is so thick that it blocks the bowel. Babies born with this problem, called meconium ileus, often need an urgent operation to remove the blockage.

What causes cystic fibrosis?

Cystic fibrosis is passed down to a child by its parents. About one in 25 people in the UK carry the cystic fibrosis gene. A person with just one gene for cystic fibrosis will not be ill from it. They may not know they have the gene at all.

A child can only be born with cystic fibrosis if both of his parents carry a gene for it. However, even if both parents have the gene, with each pregnancy there is still only a one in four chance that their child will be born with the condition (CFT nd).

What are the effects of cystic fibrosis?

Children with cystic fibrosis are particularly vulnerable to chest infections, which can pose a real threat to their health. However, treatments are improving all the time (Sharma 2013, NHS 2012), so the prospects for your baby are likely to get better as he grows.

Cystic fibrosis affects other systems in the body, too. It is difficult for children with cystic fibrosis to get nutrients from their food. They are likely to have problems with digestion and bowel movements, and may also develop diabetes.

Children with cystic fibrosis need daily treatments and have to follow a special diet. They may need to attend a lot of medical appointments and stay in hospital from time to time. So it may be difficult for a child to adjust at school and in everyday life.

The good news is that many babies with cystic fibrosis go on to enjoy a good childhood and grow up to lead full lives. It may be hard at times, but if you maintain a hopeful, positive outlook, it will really help your child (Szyndler 2005). His healthcare team will encourage him to be as active as possible (NHS 2012).

Several decades ago, most children with cystic fibrosis died very young. Today, people with cystic fibrosis are living into adulthood and the average life expectancy is increasing (Davies 2007, Sharma 2013).

What is the treatment for cystic fibrosis?

If your baby has cystic fibrosis, he should be looked after by a team with expert knowledge of the condition. The team could include a:

senior doctor (consultant) who is an expert in cystic fibrosis

specialist nurse

physiotherapist

dietitian

Your GP may play a part in his care, too (Cystic Fibrosis Trust 2011, DH 2007).

There is no cure for cystic fibrosis, but regular treatments can help to manage the condition. Frequent physiotherapy, and mist inhaled through a mask, help to clear the mucus from your child's lungs. Your child's physiotherapist can teach you how to do this at home. As your child grows up, he can probably learn to do the physiotherapy himself most of the time. Other treatments may include:

exercises to help his lungs and build stamina

a special diet and tablets to help him digest food

tablets to help his body break down mucus

regular antibiotics to ward off chest infections

Where can I find more information and support?

The Cystic Fibrosis Trust is a charity that supports anyone affected by cystic fibrosis. Advisers can help with benefits and grants, as well as medical questions. There is a telephone helpline: 0845 859 1000.

Contact a Family is a charity that can put you in touch with other parents of children with cystic fibrosis. There is a free telephone helpline: 0808 808 3555.

Last reviewed: May 2013

References

Cystic Fibrosis Trust. 2011. Standards for the clinical care of children and adults with cystic fibrosis in the UK 2001. www.cftrust.org.uk [Accessed March 2013]

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